This is a 22-year-old woman G1, referred to our unit at 13 weeks of gestation for acrania. She has no significant medical history. The sonogram confirmed the acrania. The brain tissue was present associated with an exencephaly. After extensive counseling, parents requested termination of pregnancy. Amniocentesis was performed just before the delivery. The karyotype was normal (46 XX). The spine was normal.